Case Report: Carnitine Palmitoyl Transferase II (CPT II) Deficiency

Carnitine Palmitoyl Transferase II (CPTII) is a very important enzyme that helps with the oxidation of long-chain fatty acid to produce energy. Deficiency in CPTII will lead to energy deficiency in the case of fasting and the accumulation of the long chain fatty in the body. There are three types of CPT II deficiency, the myopathic form, the severe infantile hepatocardiomuscular form and the lethal neonatal form. They are all inherited as an autosomal recessive. Diagnosis of the CPTII are 1) tandem mass spectrometry (MS/MS) in adult form and 2) CPTII polymorphism (F352C), which is linked to reducing the activity of CPTII in infantile form [1]. Glucose is the primary management and medium-chain fatty acid is an alternative due to the bypass of the CPTII enzyme in the pathway. For the prevention of CPTII deficiency are to avoid long chain fatty acid (C12-fatty acid), fasting, prolonged exercise, known triggers, and certain medications such as anti-epileptics and general anesthesia. During the rhabdomyolysis and myoglobinuria attack, it is very important to maintain hydration to avoid acute renal failure. If, however, renal failure occurs, dialysis is recommended. We present a case of a 27-year-old African American woman with the significant past medical history of CPT II deficiency leading to recurrent rhabdomyolysis and myoglobinuria. Together with all the research studies from diagnosis to treatment of CPTII deficiency will help in clinical management of patients. And this case report will add to the existing case reports of patients who have CPTII deficiency in terms of how we diagnose, how we treat, and how we prevent symptoms from re-occurring.

The Substructure of Elementary Particles Demonstrated by the I-Theory

Present studies in physics assume that elementary particles are the building blocks of all matter, and that they are zero-dimensional objects which do not occupy space. The new I-Theory predicts that elementary particles do indeed have a substructure, three dimensions, and occupy space, being composed of fundamental particles called I-particles. In this article we identify the substructural pattern of elementary particles and define the quanta of energy that form each elementary particle. We demonstrate that the substructure comprises two classes of quanta which we call “attraction quanta” and “repulsion quanta”. We create a model that defines the rest-mass energy of each elementary particle and can predict new particles. Lastly, in order to incorporate this knowledge into the contemporary models of science, a revised periodic table is proposed.

Before the Jury Is out on Cinacalcet’s Cardiovascular Effects in Hemodialysis Patients: Is Troponin a Missing Link?

Raised levels of the cardiac biomarker, Troponin I, are frequently encountered in hemodialysis patients and appear to be prognostic indicators for cardiovascular risk. Though evidence suggests that control of secondary hyperparathyroidism may reduce cardiac endpoints, the effect of the calcimimetic agent, cinacalcet, remains controversial. This retrospective study aimed at evaluating troponin levels in hemodialysis patients with severe secondary hyper parathyroidism (SHPT) who are on cinacalcet vs controls on conventional treatment. In addition, clinical outcomes including all-cause, cardiovascular morbidity and mortality were compared among both groups. A decline in Troponin I levels was observed in the cinacalcet group, this however was not translated clinically into improved survival. In fact, all-cause and cardiac mortality was similar in the two groups. Conversely, comparison of the incidence of cardiovascular events revealed lower rates in the cinacalcet group including cardiac, cerebral and peripheral vascular complications. Given some of our study limitations, further long-term, placebo-controlled trials are necessary to definitively establish the effect of cinacalet on cardiac biomarkers and ultimately its impact on clinical outcomes.

I_(sn)-I-Fréchet-Urysohn空间上的相关性质研究

提出了I_(sn)-I-Fréchet-Urysohn空间的概念,结合I_(sn)-序列空间的概念,讨论了它们之间的关系和一些相关性质,比如遗传性、连续性等,还讨论了这些空间和理想收敛中其他空间之间的关系。

Endoscopic tools for the diagnosis and evaluation of celiac disease

Celiac disease(CD)is an autoimmune disease of the small bowel induced by ingestion of wheat,rye and barley.Current guidelines indicate histological analysis on at least four duodenal biopsies as the only way to diagnose CD.These indications are based on the conception of the inability of standard endoscopy to make diagnosis of CD and/or to drive biopsy sampling.Over the last years,technology development of endoscopic devices has greatly ameliorated the accuracy of macroscopic evaluation of duodenal villous pattern,increasing the diagnostic power of endoscopy of CD.The aim of this paper is to review the new endoscopic tools and procedures proved to be useful in the diagnosis of CD,such as chromoendoscopy,Fujinon Intelligent Chromo Endoscopy,Narrow Band Imaging,Optical Coherence Tomography,Water-Immersion Technique,confocal laser endomicroscopy,high-resolution magnification endoscopy,capsule endoscopy and I-Scan technology.

<i>γ</i>-Ray Irradiation Effect on MCF Rubber Solar Cells with both Photovoltaics and Sensing Involving Semiconductors Fabricated under Magnetic and Electric Fields

For cases in which a robot with installed solar cells and a sensor operates in a nuclear reactor building or in space for extravehicular activity, we require elastic and extensible solar cells. More than two different types of sensing are also required, minimally with photovoltaics and built-in electricity. Magnetic compound fluid (MCF) rubber solar cells are made of rubber, so they are elastic and extensible as well as sensitive. To achieve flexibility and an effective photovoltaic effect, MCF rubber solar cells must include both soluble and insoluble rubbers, Fe3O4, TiO2, Na2WO4∙2H2O, etc. On the basis of this constitution, we propose a consummate fabrication process for MCF rubber solar cells. The characteristics of these cells result from the semiconductor-like role of the molecules of TiO2, Fe3O4, Ni, Na2WO4∙2H2O, polydimethylsiloxane (PDMS), natural rubber (NR), oleic acid, polyvinyl alcohol (PVA), water and magnetic cluster involved in the MCF rubber. Their tendencies can be deduced by synthesizing knowledge about the enhancement of the reverse-bias saturation current IS and the diode ideality factor N, with conventional knowledge about the semiconductor affected by γ-irradiation and the attenuation of the photon energy of γ-rays.

Clinical and Therapeutic Aspects of Inguinal Hernia in Children in the General Surgery Department of Reference Health Center in Commune I of Bamako Mali

Inguinal hernia in children is a congenital pathology in children linked to the persistence of the peritoneo-vaginal canal in children and the NüCK canal in girls;persisting into adulthood. This work aimed to study inguinal hernia in children in the general surgery department of the Reference Health Center of Commune I of the Bamako District;to determine the frequency of inguinal hernia;describe the epidemiological, clinical and therapeutic aspects of inguinal hernias and in order to evaluate the cost of treatment. This was a prospective study from April 2017 to March 2018 during which 60 children with an inguinal hernia were collected. Inguinal hernias in children represented 9.23% of patients operated on in the department. The average age was 59.23 months with extremes of one month and 180 months and a standard deviation of 49.23 years. The male sex was dominant with a sex ratio equal to 9:1. The notion of prematurity was found in 11.7% and was associated with a testicle not in place in 1.7% of cases. 41.7% of our patients were referred by a doctor and 66.7% of patients presented with painless, intermittent inguino-scrotal swelling (83.3% of cases). The hernia was discovered before one week of life in 50% of cases. Inguinal swelling was absent in 11.7% but observed intraoperatively. The inguinal hernia was unilateral in 91.7% of cases and 70% of hernias were discovered by the parents during pushing efforts. The inguinal hernia was located on the right in 61.7% of cases. The hernia swelling was soft, painless, impulsive and reducible in 78.3% of cases. The swelling was inguino-scrotal in 58.3% of cases. The hernia was simple in 95% of patients and hernial strangulation was observed in 3 cases or 5% of cases. 98.3% of patients were ASA I. The treatment was carried out openly in all our patients including closure of the vaginal peritoneal canal in 95% of cases under general anesthesia in 98.3% of cases. The morbidity rate was 8.4% (surgical site infection: 6.7% of cases and hematoma: 1.7%). The immediate consequences were simple in 96.6% of cases. No cases of recurrence occurred during the 6 months after the intervention. The average cost of care was estimated at 69,743 FCFA.

Design Method for Optimizing the Interactive Interface of Live Broadcasting Platform for the Elderly Users

In the era of network live broadcasting for everyone,the development of live broadcasting platforms is also more intelligent and diversified.However,in the face of a large group of elderly users,the interface interaction design mode used is still mainly based on the interaction mode for young groups,and is not designed for elderly users.Therefore,a design method for optimizing the interaction interface of live broadcasting platform for elderly users was proposed in this study.Firstly,the case study method and Delphi expert survey method were used to determine the design needs of elderly users and the design mode was analysed.Secondly,the orthogonal design principle was used to design a test sample of the interactive interface of live broadcasting platform applicable for the elderly users,and then a user evaluation system was established to calculate the weights of the design elements using hierarchical analysis,and then the predictive relationship between the design mode of the interactive interface of live broadcasting platform and the elderly users was established by Quantitative Theory I.Finally,Genetic Algorithm was applied to generate the optimized design scheme.The results showed that the design method based on the Genetic Algorithm and the combination of Quantitative Theory can scientifically and effectively optimize the design of the interactive interface of the live broadcasting platform for the elderly users,and improve the experience of the elderly users.

Can the Hypothetical Protons Emitted by the Shroud’s Man Furnish an I(z) Correlation?

Recently, we have investigated the hypothesis radiative demonstrating that the two penetrated thicknesses (in air and linen) are not compatible with a single energy of the protons. Furthermore, we deduced that the distribution of energy, released by the above particles, on the burial linen has not a linear trend when the body-burial linen distance changes. Now, in this article we want to deduce the I(z) relationship, between the Image Intensity of the colour produced by protons on a linen and the z distance from the source (of Protons) and the same linen. To achieve the result in an analytical form and make a comparison with the same function extracted from the Shroud, we used the empirical expression Range-Energy for protons in air of Wilson-Brobeck. Thus, we obtain a result I(z) = Im [1 − (z/R)5/9] that is different from the one extracted from the Turin Linen I(z) = IM (1 − z/R0). We have also the same information using the Range-Energy curves for protons of Rogozinski. The result is negative for the radiative hypothesis that is unable to produce the Shroud Body Image. Therefore, to investigate the above unknown process of formation, it is necessary to think about another one.

Phase Transitions Governed by the Fifth Power of the Golden Mean and Beyond

In this contribution results from different disciplines of science were compared to show their intimate interweaving with each other having in common the golden ratio φ respectively its fifth power φ5. The research fields cover model calculations of statistical physics associated with phase transitions, the quantum probability of two particles, new physics of everything suggested by the information relativity theory (IRT) including explanations of cosmological relevance, the ε-infinity theory, superconductivity, and the Tammes problem of the largest diameter of N non-overlapping circles on the surface of a sphere with its connection to viral morphology and crystallography. Finally, Fibonacci anyons proposed for topological quantum computation (TQC) were briefly described in comparison to the recently formulated reverse Fibonacci approach using the Janičko number sequence. An architecture applicable for a quantum computer is proposed consisting of 13-step twisted microtubules similar to tubulin microtubules of living matter. Most topics point to the omnipresence of the golden mean as the numerical dominator of our world.

Comparison of Type I Error Rates of Siegel-Tukey and Savage Tests among Non-Parametric Tests

This study aimed to examine the performance of the Siegel-Tukey and Savage tests on data sets with heterogeneous variances. The analysis, considering Normal, Platykurtic, and Skewed distributions and a standard deviation ratio of 1, was conducted for both small and large sample sizes. For small sample sizes, two main categories were established: equal and different sample sizes. Analyses were performed using Monte Carlo simulations with 20,000 repetitions for each scenario, and the simulations were evaluated using SAS software. For small sample sizes, the I. type error rate of the Siegel-Tukey test generally ranged from 0.045 to 0.055, while the I. type error rate of the Savage test was observed to range from 0.016 to 0.041. Similar trends were observed for Platykurtic and Skewed distributions. In scenarios with different sample sizes, the Savage test generally exhibited lower I. type error rates. For large sample sizes, two main categories were established: equal and different sample sizes. For large sample sizes, the I. type error rate of the Siegel-Tukey test ranged from 0.047 to 0.052, while the I. type error rate of the Savage test ranged from 0.043 to 0.051. In cases of equal sample sizes, both tests generally had lower error rates, with the Savage test providing more consistent results for large sample sizes. In conclusion, it was determined that the Savage test provides lower I. type error rates for small sample sizes and that both tests have similar error rates for large sample sizes. These findings suggest that the Savage test could be a more reliable option when analyzing variance differences.

Mark VIe控制系统在燃驱压缩机组控制系统升级改造中的应用

针对当前集成了不同厂家几套子系统的一些燃驱压缩机组控制系统结构复杂、维护成本高等问题,应用基于Mark VIe控制平台来升级改造该系统。以某改造项目为例,应用双冗余Mark VIe控制系统架构,Mark VIe SIL3模件作为紧急停机和火气保护系统的硬件,同时采用Mark VIe ControlST统一软件平台。结果表明:新控制系统采用了一体化硬件、软件设计,提高了机组控制系统的安全可靠性,有利于日常运行维护,而且新系统具有在线监测功能,有助于系统故障排查。

Mitochondrial Signaling in Hypoxia

This paper focuses on a bioenergetic mechanism responding to hypoxia. This response involves hypoxia-induced reprogramming of respiratory chain function and switching from oxidation of complex I (NAD-related substrates) to complex II (succinate oxidation). Transient, reversible, compensatory activation of respiratory chain complex II is a major mechanism of urgent adaptation to hypoxia, which is necessary for 1) succinate-related energy synthesis in the conditions of oxygen shortage and formation of urgent resistance;2) succinate-related stabilization of HIF-1α and initiation of its transcriptional activity related with formation of long-term adaptation;3) succinate-dependent activation of the succinate-specific receptor GPR91. Thus, mitochondria perform a signaling function with succinate as a signaling molecule. Effects of succinate in hypoxia occur at three levels, intramitochondrial, intracellular and intercellular. In these settings, succinate displays antihypoxic activity. The review is focused on tactics and strategy for development of the antihypoxic defense and antihypoxants with energotropic properties.

Mitochondrial Signaling in Hypoxia

This paper focuses on a bioenergetic mechanism responding to hypoxia. This response involves hypoxia-induced reprogramming of respiratory chain function and switching from oxidation of complex I (NAD-related substrates) to complex II (succinate oxidation). Transient, reversible, compensatory activation of respiratory chain complex II is a major mechanism of urgent adaptation to hypoxia, which is necessary for 1) succinate-related energy synthesis in the conditions of oxygen shortage and formation of urgent resistance;2) succinate-related stabilization of HIF-1α and initiation of its transcriptional activity related with formation of long-term adaptation;3) succinate-dependent activation of the succinate-specific receptor GPR91. Thus, mitochondria perform a signaling function with succinate as a signaling molecule. Effects of succinate in hypoxia occur at three levels, intramitochondrial, intracellular and intercellular. In these settings, succinate displays antihypoxic activitie. The review is focused on tactics and strategy for development of the antihypoxic defense and antihypoxants with energotropic properties.

Physiologic Type Reconstruction in Complicated Corrosive Strictures of Upper Gastrointestinal Segment

Objectives: The main steps for physiologic type reconstruction in 50 complicated corrosive strictures of upper alimentary tract are presented. Methods: In successive developed gastric outlet and esophageal strictures a limited Billroth I resection (in 9) or conversion a prior precolic GEA in such anastomosis (in 5) and middle or total gastrectomies (in 3) were performed. A second stage substernal by-pass with isoperistaltic transverse colon segment was done 6 - 12 weeks later. In all but one instances the graft was implanted high in the gastric stump. In extensive burned and retracted such lesion (in 3) a similar by-pass was carried out but the lower anastomosis was done with the not involved prepyloric segement. In concomittant antropyloric and esophageal strictures in 11 young, good risk patients, a limited Billroth I resction and simultaneous colonic bypass was used. In case of accompanied respiratory fistula (in 4) exclusion by-pass was useful for both lesions. The associated pyloric stricture (in 3) was solved at the same time. Side-to-end pharyngocolostomy was used in 4 high thoracocervical strictures. In 8 previously perforated strictures the by-ass was performed 2 months later. Reults: The overall mortality was 4%. The postoperative morbidity was low (8%). All cervical leaks closed spontaneously. Particular late complications required revisional surgery in 12, 5% of cases. Conclusion: In complicated corrosive strictures (esophageal, gastric, fistulas) limited Billoth I resection, isoperistaltic colon by-pass with high gastrocolic anastomosis, good gastric drainage and maintenance of the duodenum in gastrointestinal continuity are the main factors to achieve the best functional results.

重楼皂苷Ⅰ预防给药对心肌缺血再灌注损伤大鼠调节PI3K/AKT信号通路的作用研究

目的 从磷脂酰肌醇-3-激酶(PI3K)/蛋白激酶B(AKT)信号通路探讨重楼皂苷I预防给药对心肌缺血再灌注损伤(MI/IR)大鼠的干预机制。方法 将购买的72只SPF级SD大鼠按照随机数字法分为假手术组、模型组、阿司匹林组、重楼皂苷I低、中和高剂量组,每组12只。分组后即给予相应药物干预2周,2周后构建MI/IR模型。模型构建成功24 h后先采用动物彩色多普勒超声仪检测心脏功能,后处死大鼠收集相关组织采用HE染色观察心肌病理学、TTC法检测心肌梗死面积,试剂盒检测心功能指标[乳酸脱氢酶(LDH)、肌酸激酶同工酶MB(CK-MB)和谷草转氨酶(AST),抗氧化指标丙二醛(MDA)、超氧化物歧化酶(SOD)和谷胱甘肽(GSH)],TUNNEL染色检测心肌细胞凋亡特点,Western blot检测心肌PI3K、AKT、B细胞淋巴瘤/因子2(Bcl-2)、Bcl-2相关蛋白(Bax)蛋白表达。结果 假手术组心肌纤维排列整齐、无水肿,模型组有心肌纤维断裂,重楼皂苷I低、中、高剂量组和阿司匹林组明显改善心肌纤维断裂情况。与假手术组相比,模型组大鼠心肌梗死面积、LVEDP、LDH、CK-MB、AST和MDA明显升高(P<0.05),LVDP、+dp/dtmax、-dp/dtmax、SOD和GSH明显降低(P<0.05);相较于模型组,阿司匹林组和重楼皂苷I低、中、高剂量组干预后,心肌梗死面积、LVEDP、LDH、CK-MB、AST和MDA明显降低(P<0.05),LVDP、+dp/dtmax、-dp/dtmax、SOD和GSH则明显升高(P<0.05)。Tunnel染色可见,假手术组几乎没有心肌细胞凋亡,而模型组呈现出明显的大量的细胞凋亡;与模型组相比,阿司匹林组和重楼皂苷I低、中、高剂量组凋亡情况明显好转。此外,与假手术组相比,模型组大鼠心肌PI3K、AKT和Bcl-2蛋白表达均明显降低,Bax明显升高(均P<0.05);相较于模型组,重楼皂苷I低、中、高剂量组以及阿司匹林组PI3K、AKT和Bcl-2蛋白表达均明显升高,Bax蛋白表达明显降低(均P<0.05)。结论 重楼皂苷I对心肌缺血再灌注损伤有一定的预防作用,其作用机理可能与其能够调节PI3K/AKT信号通路有关。

How Water-Development Nexus Can Reunite Central Asia?

The following article has been retracted due to the fact that it cannot be accepted by the author’s university as a scientific peer-reviewed publication. The Editorial Board takes a very strong respect to the author’s situation on this matter. This paper published in World Journal of Engineering and Technology Vol.2 No.3B, September 2014, has been removed from this site.

Persimmon leaf flavonoid induces brain ischemic tolerance in mice

The persimmon leaf has been shown to improve cerebral ischemic outcomes; however, its mechanism of action remains unclear. In this study, mice were subjected to 10 minutes of ischemic preconditioning, and persimmon leaf flavonoid was orally administered for 5 days. Results showed that the persimmon leaf fiavonoid significantly improved the content of tissue type plasminogen activator and 6-keto prostaglandin-F1 a in the cerebral cortex, decreased the content of thromboxane B2, and reduced the content of plasminogen activator inhibitor-1 in mice. Following optical microscopy, persimmon leaf flavonoid was also shown to reduce cell swelling and nuclear hyperchromatism in the cerebral cortex and hippocampus of mice. These results suggested that persimmon leaf fiavonoid can effectively inhibit brain thrombosis, improve blood supply to the brain and relieve ischemia-induced pathological damage, resulting in brain ischemic tolerance.

基于改进SVIT算法的非侵入式居民负荷监测方法

针对居民侧异常用电负荷对用户用电安全有重大影响的问题,提出了一种基于改进SVIT的非侵入式负荷监测方法。该方法从用户总线处对居民家庭用电负荷投切时的V-I数据进行特征提取;对提取出的V-I轨迹特征进行二进制化处理并绘制成V-I轨迹图像;利用改进的与三重注意力机制(Triplet Attention)相结合的SVIT的特征提取网络对其进行特征提取与映射;在此基础上,将处理后生成的新的特征向量进行聚类形成特征检索数据库,以识别出不在该特征检索数据库中的用电器V-I轨迹样本。通过利用PLAID数据集进行仿真实验并分析,验证了模型的有效性以及算法的优越性。

Associations with Organ Involvement and Autoantibodies in Systemic Sclerosis: Results from the Canadian Scleroderma Research Group (CSRG)

Objective: Serum from SSc patients was analyzed centrally to determine ANA patterns and extractable nuclear antigens (ENAs) between lcSSc and dcSSc and associations with organ involvement. Methods: 1145 SSc patients had ANA and ENA analyzed by indirect immunofluorescence on HEp-2 substrate at a screening serum dilution of 1/160. Most ENA antibodies [Sm. U1-RNP, Ro52, SS-A/Ro60, topoisomeraseI (Topo1), SS-B/La, chromatin, ribosomal P and Jo1] were measured by laser bead immunoassay;and RNA polymerase III (RNAP) by ELISA. Results: ANA was positive in 95% (same in lcSSc, and dcSSc). Centromere pattern was present in 34%, speckled 22%, nucleolar 18%, homogeneous and speckled (H&S) 16%, multiple nuclear dots 6%. Anti-centromere Ab (ACA) occurred in 46% of lcSSc and 11% of dcSSc (P = 0.0001). ENAs that differed between lcSSc and dcSSc subsets were Topo1 (OR 2.4, P = 0.0001) and RNAP (OR 5.6, P 0.0001) more common in dcSSc. Overall, 15% had positive Topo1;usually with a H&S pattern (67%);Topo1 was associated with ILD on CXR (OR 2.3;95% CI 1.5 - 3.5) and HRCT (OR 3.8;95% CI 1.8 - 8.2). RNAP occurred in 18.5% (35.4% in dcSSc vs. 8.9% in lcSSc). Scleroderma renal crisis (SRC) was 13 times more likely if RNAP positive;P = 0.0001. ACA was only weakly associated with sPAP > 50 mmHg (OR 1.8;95%CI 1.1 - 3.0). Conclusion: ANA homogeneous pattern alone is rare in SSc;ACA was significantly more common in lcSSc. Many ENAs are equal in lcSSc and dcSSc except RNAP and Topo1. RNAP has the highest OR of SRC. Topo1 is less strongly associated with ILD. Abstract word count: 249, Body word count 1246, Figures 2, Tables 2. Key Messages: 1) 95% of SSc has a positive ANA and ANA patterns in SSc include centromere, nucleolar, and homogeneous and speckled together;2) Most ENAs are equal in both dcSSc and lcSSc except anti RNA polymerase III and topoisomerase I;3) RNA polymerase III has the highest association (odds ratio) with scleroderma renal crisis, topoisomerase I is associated with interstitial lung disease;whereas anticentromere was not associated with elevated pulmonary arterial pressures on echocardiogram.